Ewing sarcoma

Background[1]

Distribution of Ewing's sarcoma: Most frequent locations are the large long bones and the pelvis.

Highly aggressive malignant bone or soft tissue tumor arising from cells of the neuroectoderm
One-fourth of patients will have metastasis at the time of presentation
- It is assumed that almost all patients have subclinical metastasis
Presence and location of metastasis is the major prognostic factor for survival
Metastasis is most frequently seen in bone (spine most common) and lungs
Around 70 to 80 percent survival in those with non-metastatic disease
More common in males

Constant pain for months over site of malignancy
- Worse with exercise and at night
Site will be edematous and tender to palpation
- Mass can sometimes be appreciated
Most often found in pelvis, axial skeleton, or diaphysis of femur with a small minority arising in soft tissues
Pelvic tumors are more regularly associated with metastatic disease
Fever, weight loss, fatigue, night sweats occur in less than 20 percent of patients
Pathological fractures can occur

X-ray of a child with Ewing's sarcoma of the tibia

MRI showing Ewing's sarcoma of the left hip (white area)

Appearance on plain radiographs often described as “onion peel” or “moth-eaten” appearance referring to the respective periosteal bone formation and osseous destruction
LDH is a prognostic factor
Definitive diagnosis is made with biopsy
Work-up often includes PET scan for metastatic disease
t(11;22) translocation is often seen

Niederhuber, John E., et al. “Sarcomas.” Abeloff's Clinical Oncology E-Book. Elsevier Health Sciences, 2019, pp. 1604 – 1654.e8.

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